title: Rates of progression of weight and forced vital capacity as relevant measurement to adapt amyotrophic lateral sclerosis management for patient Result of a French multicentre cohort survey.
abstract: OBJECTIVE
  To compare survival, to describe the progression of anthropometry, pulmonary capacity and functioning in ALS (Amyotrophic Lateral Sclerosis) and to identify the most relevant variables to adapt ALS management for patients.
  
  
  METHODS
  A cohort study was performed in French ALS centres between January 2003 and July 2005. Eligible patients were treated by Riluzole and had a slow vital capacity (SVC) or a forced vital capacity (FVC) at least equal to 60%. Demographic, medical and ALS characteristics were registered. Manual Muscular Testing (MMT) and ALS Functional Rating Scale (ALSFRS) were performed. Kaplan Meier method was used to analyse survival. ALS progression was measured by the percentage weight, FVC, SVC, MMT and ALSFRS loss and was analysed as longitudinal data using mixed model.
  
  
  RESULTS
  Three hundred and eighty three patients were included. The median survival since ALS diagnosis was 2.34 years (95%CI 2.10-2.65). Mixed model analyses revealed a more significant worsening progression of weight and FVC loss for bulbar onset. The drop of ALSFRS and SVC is similar whatever the ALS forms.
  
  
  CONCLUSIONS
  Rates of progression of weight and FVC should be regularly watched over to support neurologists to adapt ALS management for patients.
authors.affiliation: Department of Neurology, University Hospital of Clermont-Ferrand, France.
authors.forename: P
authors.initials: P
authors.lastname: Clavelou
authors.forename: M
authors.initials: M
authors.lastname: Blanquet
authors.forename: F
authors.initials: F
authors.lastname: Peyrol
authors.forename: L
authors.initials: L
authors.lastname: Ouchchane
authors.forename: L
authors.initials: L
authors.lastname: Gerbaud
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000188:drug therapy / Q000401:mortality / Q000503:physiopathology*; D015992:Body Mass Index; D001835:Body Weight / Q000187:drug effects / Q000502:physiology*; D015331:Cohort Studies; D018450:Disease Progression; D005260:Female; D005602:France; D006801:Humans; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination; D018696:Neuroprotective Agents / Q000627:therapeutic use; D012189:Retrospective Studies; D019782:Riluzole / Q000627:therapeutic use; D015996:Survival Rate; D013997:Time Factors; D014797:Vital Capacity / Q000187:drug effects / Q000502:physiology*
chemical_list: D018696:Neuroprotective Agents; D019782:Riluzole
keywords: Amyotrophic lateral sclerosis; Forced vital capacity; Mixed models; Motor neurone disease; Prognosis factor; Survival